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Background: Mitral valve prolapse (MVP) is responsible for a considerable disease burden but is widely heterogeneous. The lack of a comprehensive prognostic instrument covering the entire MVP spectrum, encompassing the quantified consequent degenerative mitral regurgitation (DMR), hinders clinical management and therapeutic trials. Methods: The new Mitral Regurgitation International Database Quantitative (MIDA-Q) registry enrolled 8187 consecutive patients (ages 63±16 years, 47% women, follow-up 5.5±3.3 years) first diagnosed with isolated MVP, without or with DMR quantified prospectively (measuring effective regurgitant orifice [ERO] and regurgitant volume) in routine practice of 5 tertiary care centers from North America, Europe, and the Middle East. The MIDA-Q score ranges from 0 to 15 by accumulating guideline-based risk factors and DMR severity. Long-term survival under medical management was the primary outcome end point. Results: MVP was associated with DMR absent/mild (ERO 2 ) in 50%, moderate (ERO 20–40 mm 2 ) in 25%, and severe or higher (ERO ≥40 mm 2 ) in 25%, with mean ERO 24±24 mm 2 , regurgitant volume 37±35 mL. Median MIDA-Q score was 4 with a wide distribution (10%–90% range, 0–9). MIDA-Q score was higher in patients with EuroScore II ≥1% versus P < 0.0001) but with wide overlap (10%–90% range, 4–11 versus 0–7) and mediocre correlation ( R 2 0.18). Five-year survival under medical management was strongly associated with MIDA-Q score, 97±1% with score 0, 95±1% with score 1 to 2, 82±1% with score 3 to 4, 67±1% with score 5 to 6, 60±1% with score 7 to 8, 44±1% with score 9 to 10, 35±1% with score 11 to 12, and 5±4% with MIDA-Q score ≥13, with hazard ratio 1.31 [1.29–1.33] per 1-point increment. Excess mortality with higher MIDA-Q scores persisted after adjustment for age, sex, and EuroScore II (adjusted hazard ratio, 1.13 [1.11–1.15] per 1-point increment). Subgroup analysis showed persistent association of MIDA-Q score with mortality in all possible subsets, in particular, with EuroScore IIP P Conclusions: This large, international cohort of isolated MVP, with prospective DMR quantification in routine practice, demonstrates the wide range of risk factor accumulation and considerable heterogeneity of outcomes after MVP diagnosis. The MIDA-Q score is strongly, independently, and incrementally associated with long-term survival after MVP diagnosis, irrespective of presentation, and is therefore a crucial prognostic instrument for risk stratification, clinical trials, and management of patients diagnosed with all forms of MVP.

Aims The current guidelines recommend aortic valve intervention in patients with severe aortic regurgitation (AR) with the onset of symptoms, left ventricular enlargement, or systolic dysfunction. Recent studies have suggested that we might be missing the window of early intervention in a significant number of patients by following the guidelines. Methods and results The overarching goal was to determine if machine learning (ML)-based algorithms could be trained to identify patients at risk for death from AR independent of aortic valve replacement (AVR). Models were trained with five-fold cross-validation on a dataset of 1035 patients, and performance was reported on an independent dataset of 207 patients. Optimal predictive performance was observed with a conditional random survival forest model. A subset of 19/41 variables was selected for inclusion in the final model. Variable selection was performed with 10-fold cross-validation using random survival forest model. The top variables included were age, body surface area, body mass index, diastolic blood pressure, New York Heart Association class, AVR, comorbidities, ejection fraction, end-diastolic volume, and end-systolic dimension, and the relative variable importance averaged across five splits of cross-validation in each repeat were evaluated. The concordance index for predicting survival of the best-performing model was 0.84 at 1 year, 0.86 at 2 years, and 0.87 overall, respectively. Conclusion Using common echocardiographic parameters and patient characteristics, we successfully trained multiple ML models to predict survival in patients with severe AR. This technique could be applied to identify high-risk patients who would benefit from early intervention, thereby improving patient outcomes.

The bicuspid aortic valve (BAV) is associated with significant aortic valve dysfunction. We aimed to study Mexican patients with BAV to assess phenotypic expressions of BAV, and associations of valvulopathy and aortopathy and their predictors. A cross-sectional, retrospective study was designed. The patients were divided according to (1) normally or minimally dysfunctional BAV, (2) predominant aortic regurgitation (AR), and (3) predominant aortic stenosis (AS). A total of 189 patients were included with a high prevalence of males (68%). The overall median age was 42 (23 to 52) years. The distribution of fused BAV phenotypes was right-left fusion (77%), right noncoronary fusion (17%) and left noncoronary fusion (6%). AS-predominant group was the oldest with a high prevalence of hypertension, type 2 diabetes mellitus (T2DM), and raphe. In multivariable analysis T2DM (odds ratio [OR] 10.5 [95% confidence interval (CI) 2.1 to 52], p0.01) and presence of raphe (OR 2.58 [95% CI 1.02 to 6.52], p = 0.04) were independently associated with AS. The AR-predominant group was composed mostly of males with significantly fewer cardiovascular risk factors. Male gender (OR 2.84 [95%CI 121 to 6.68], p = 0.01) and aortic dilatation (OR 3.58 [95% CI 1.73 to 7.39], p0.01) were associated with AR-predominance in multivariable analysis. Aortic dilatation was associated with age (OR 1.03 [95% CI 1.008 to 1.05], p0.01) and AR (OR 4.31 [95% CI 2.05 to 9.06], p0.01). Independent factors associated with the root phenotype were male gender (OR 12.4 [95% CI 1.6 to 95], p0.01) and AR (OR 5.25 [95% CI 2.18 to 12.6], p0.01).In conclusion, in a mestizo population, the distribution of BAV-fused phenotypes was similar to European and North American populations, the presence of raphe and T2DM were independently associated with AS-predominance, and male gender and aortic dilatation were independently associated with AR-predominance.

Mitral transcatheter edge-to-edge repair (MTEER) is an established therapeutic approach for mitral regurgitation (MR). Functional mitral regurgitation originating from atrial myopathy (A-FMR) has been described.We sought to assess the clinical, echocardiographic and hemodynamic considerations in A-FMR patients undergoing MTEER.From 2014 to 2020, patients undergoing MTEER for degenerative MR (DMR), functional MR (FMR), and mixed MR were assessed. A-FMR was defined by the presence of MR moderate in severity; left ventricular (LV) ejection fraction (LVEF) ≥ 50%; and severe left atrial (LA) enlargement in the absence of LV dysfunction, leaflet pathology, or LV tethering. The diagnosis of A-FMR (vs. ventricular-FMR [V-FMR]) was confirmed by three independent echocardiographers. Baseline characteristics, procedural outcomes as well as clinical and echocardiographic follow-up are reported. Device success was defined as final MR grade ≤ moderate; MR reduction ≥1 grade; and final transmitral gradient5 mmHg.306 patients underwent MTEER, including DMR (62%), FMR (19%), and mixed MR (19%). FMR cases included 37 (63.8%) V-FMR and 21 (36.2%) A-FMR. Tricuspid regurgitation (≥ moderate) was higher in A-FMR (80.1%) compared to V-FMR (54%) and DMR (42%). Device success did not significantly differ between A-FMR and V-FMR (57% vs. 73%, p = 0.34) or DMR (57% vs. 64%, p = 1.0). The A-FMR cohort was less likely to achieve ≥3 grades of MR reduction compared to V-FMR (19% vs. 54%, p = 0.01) and DMR (19% vs. 49.7%, p = 0.01). Patients with V-FMR and DMR demonstrated significant reductions in mean left atrial pressure (LAP) and peak LA V-wave, though A-FMR did not (LAP -0.24 ± 4.9, p = 0.83; peak V-wave -1.76 ± 9.1, p = 0.39). In follow-up, echocardiographic and clinical outcomes were similar.In patients undergoing MTEER, A-FMR represents one-third of FMR cases. A-FMR demonstrates similar procedural success but blunted acute hemodynamic responses compared with DMR and V-FMR following MTEER. Dedicated studies specifically considering A-FMR are needed to discern the optimal therapeutic approaches.

ObjectiveNon-bacterial thrombotic endocarditis (NBTE) is a syndrome characterised by cardiac valve vegetations and/or thickening due to non-infective mechanisms. Nowadays, a premortem diagnosis of NBTE is possible based on echocardiographic findings. Therefore, to better characterise this disease, we performed a contemporary review of the epidemiology, demographics, diagnosis and clinical outcomes of these patients.MethodsAdults with a diagnosis of NBTE seen within the Mayo Clinic Enterprise from December 2014 to December 2021 were included. NBTE diagnosis was identified by clinicians representing at least two specialties including cardiology, infectious diseases, rheumatology and oncology. Patients with positive blood cultures, infective endocarditis, culture-negative endocarditis and denial of research authorisation were excluded. All patients had a 1-year follow-up.ResultsForty-eight cases were identified; mean age was 60.0±13.8 years, 75% were female. The most prevalent comorbidities were malignancy (52.1%) and connective tissue disease (37.5%). Valvular abnormalities included 41 (85.4%) patients with vegetations, 43 (89.6%) patients with thickening and 26 (54.2%) with moderate to severe regurgitation. Thirty-eight (79.2%) patients had an embolic event (stroke in 26 (54.2%) patients) within 1 month of NBTE diagnosis and 16 (33.3%) patients died within 1 year of NBTE diagnosis. Metastatic tumours and lung cancer were associated with 1-year all-cause mortality (p=0.0017 and p=0.0004, respectively).ConclusionsNBTE was more prevalent in females and embolic complications were the most frequent clinical finding. Overall, patients with NBTE had a poor prognosis, particularly in those with lung cancer or metastatic tumours. Further studies in patients with NBTE are needed given its morbidity and mortality.

Patients who need a prosthetic aortic heart valve may decide, working with their cardiologist and cardiac surgeon, among a variety of options: surgical or transcatheter approach, bioprosthetic or mechanical valve, or a Ross procedure if suitable to their age and anatomy. This review article examines the evidence for survival benefit with mechanical aortic valves, discusses bioprosthetic structural valve degeneration and its consequences, and considers the risks of redo aortic valve surgery or subsequent valve-in-valve (ViV) transcatheter intervention. It highlights the unique characteristics of the On-X aortic valve, including the US Food and Drug Administration approved and American College of Cardiology/American Heart Association guideline supported reduced anticoagulation target INR of 1.5 to 2.0, and discusses the PROACT Xa trial comparing apixaban vs warfarin anticoagulation. The choice of prosthetic valve should be individualized, carefully considering each patient's unique circumstances. In that context, the On-X aortic valve offers a potential lifetime solution without need for a repeat operation, while minimizing the risks of long-term anticoagulation. In an era of enthusiasm for bioprosthetic and transcatheter-based approaches, the option of a second-generation bileaflet mechanical valve with optimized hemodynamics-the On-X aortic valve-may well align with patient expectations.

AIMS: Mitral valve prolapse (MVP) is a common valvular heart disease with a prevalence of >2% in the general adult population. Despite this high incidence, there is a limited understanding of the molecular mechanism of this disease, and no medical therapy is available for this disease. We aimed to elucidate the genetic basis of MVP in order to better understand this complex disorder.METHODS AND RESULTS: We performed a meta-analysis of six genome-wide association studies that included 4884 cases and 434 649 controls. We identified 14 loci associated with MVP in our primary analysis and 2 additional loci associated with a subset of the samples that additionally underwent mitral valve surgery. Integration of epigenetic, transcriptional, and proteomic data identified candidate MVP genes including LMCD1, SPTBN1, LTBP2, TGFB2, NMB, and ALPK3. We created a polygenic risk score (PRS) for MVP and showed an improved MVP risk prediction beyond age, sex, and clinical risk factors.CONCLUSION: We identified 14 genetic loci that are associated with MVP. Multiple analyses identified candidate genes including two transforming growth factor-β signalling molecules and spectrin β. We present the first PRS for MVP that could eventually aid risk stratification of patients for MVP screening in a clinical setting. These findings advance our understanding of this common valvular heart disease and may reveal novel therapeutic targets for intervention.KEY QUESTION: Expand our understanding of the genetic basis for mitral valve prolapse (MVP). Uncover relevant pathways and target genes for MVP pathophysiology. Leverage genetic data for MVP risk prediction.KEY FINDING: Sixteen genetic loci were significantly associated with MVP, including 13 novel loci. Interesting target genes at these loci included LTBP2, TGFB2, ALKP3, BAG3, RBM20, and SPTBN1. A risk score including clinical factors and a polygenic risk score, performed best at predicting MVP, with an area under the receiver operating characteristics curve of 0.677.TAKE-HOME MESSAGE: Mitral valve prolapse has a polygenic basis: many genetic variants cumulatively influence pre-disposition for disease. Disease risk may be modulated via changes to transforming growth factor-β signalling, the cytoskeleton, as well as cardiomyopathy pathways. Polygenic risk scores could enhance the MVP risk prediction.

Cleft-like indentations (CLIs) of the mitral valve (MV) are best assessed with three-dimensional (3D) transesophageal echocardiography (TEE). The present study examined the prevalence, characteristics, and surgical effect of MV CLIs in patients with hypertrophic cardiomyopathy (HCM).Prospective, observational, case-control study.Tertiary medical center.The study comprised 90 patients with HCM undergoing myectomy and 59 patients undergoing cardiac surgery for non-MV related indications.Intraoperative 3D TEE was used to evaluate the presence and characteristics of MV CLIs compared, with a random control group of 59 patients undergoing cardiac surgery for non-MV related indications. Ninety patients with HCM (mean age 54.8 ± 13.3 y, 67.8% male) were compared with 59 control patients (mean age 67 ± 12.7 y, 79.7% male). Three-dimensional TEE images were interpreted by consensus of two experienced echocardiographers. At least one MV CLI was present in 84 patients with HCM (93.3%), compared with 23 control patients (39%; p0.01). Compared with control patients, patients with HCM were more likely to have deep MV CLIs (85.6% v 25.4%; p0.01) and ≥2 CLIs (52.2% v 26.1%; p = 0.02). Six HCM patients (7%) appeared to have true congenital posterior leaflet clefts versus 0% in control patients (p = 0.08). Preoperative mitral regurgitation severity and jet direction were not associated with the presence of deep or multiple MV CLIs (all p0.2). None of the MV CLIs in the HCM group required MV surgical intervention or second pump runs for MV regurgitation correction after myectomy.Deep and multiple MV CLIs are common in patients with HCM undergoing septal myectomy, including possible true posterior clefts, but they are not associated with the premyectomy severity of mitral regurgitation or jet direction, and do not result in surgical MV intervention.

AimsIndications for surgery in patients with degenerative mitral regurgitation (DMR) are increasingly liberal in all clinical guidelines but the role of secondary outcome determinants (left atrial volume index ≥60 mL/m2, atrial fibrillation, pulmonary artery systolic pressure ≥50 mmHg and moderate to severe tricuspid regurgitation) and their impact on post-operative outcome remain disputed. Whether these secondary outcome markers are just reflective of the DMR severity or intrinsically affect survival after DMR surgery is uncertain and may have critical importance in the management of patients with DMR. To address these gaps of knowledge the present study gathered a large cohort of patients with quantified DMR, accounted for the number of secondary outcome markers and examined their independent impact on survival after surgical correction of the DMR.Methods and resultsThe Mitral Regurgitation International DAtabase-Quantitative registry includes patients with isolated DMR from centres across North America, Europe, and the Middle East. Patient enrolment extended from January 2003 to January 2020. All patients undergoing mitral valve surgery within 1 year of registry enrolment were selected. A total of 2276 patients [65 (55–73) years, 32% male] across five centres met study eligibility criteria. Over a median follow-up of 5.6 (3.6 to 8.7) years, 278 patients (12.2%) died. In a comprehensive multivariable Cox regression model adjusted for age, EuroSCORE II, symptoms, left ventricular ejection fraction (LVEF), left ventricular end-systolic diameter (LV ESD) and DMR severity, the number of secondary outcome determinants was independently associated with post-operative all-cause mortality, with adjusted hazard ratios of 1.56 [95% confidence interval (CI): 1.11–2.20, P = 0.011], 1.78 (95% CI: 1.23–2.58, P = 0.002) and 2.58 (95% CI: 1.73–3.83, P < 0.0001) for patients with one, two, and three or four secondary outcome determinants, respectively. A model incorporating the number of secondary outcome determinants demonstrated a higher C-index and was significantly more concordant with post-operative mortality than models incorporating traditional Class I indications alone [the presence of symptoms (P = 0.0003), or LVEF ≤60% (P = 0.006), or LV ESD ≥40 mm (P = 0.014)], while there was no significant difference in concordance observed compared with a model that incorporated the number of Class I indications for surgery combined (P = 0.71).ConclusionIn this large cohort of patients treated surgically for DMR, the presence and number of secondary outcome determinants was independently associated with post-surgical survival and demonstrated better outcome discrimination than traditional Class I indications for surgery. Randomised controlled trials are needed to determine if patients with severe DMR who demonstrate a cardiac phenotype with an increasing number of secondary outcome determinants would benefit from earlier surgery.

Objectives The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). Background Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. Methods A cohort of 595 (278 women, mean age 61 ± 16 years) consecutive patients with isolated MVP, with comprehensive clinical, rhythmic, Doppler echocardiographic, and consistent MAD assessment, were examined. MAD prevalence, associated MVP phenotypes, and outcomes (survival, clinical arrhythmic events) starting at diagnostic echocardiography were analyzed. To balance important baseline differences, propensity scoring matching was conducted among patients with and those without MAD. Results The presence of MAD was common (n = 186 [31%]) in patients with MVP, generally in younger patients, and was not random but was independently associated with severe myxomatous disease involving bileaflet MVP and marked leaflet redundancy (both P ≤ 0.0002). The presence of MAD was also independently associated with a larger left ventricle (P = 0.005). Age-matched cohort survival after MVP diagnosis was not worse with MAD (10-year survival 93% ± 2% for patients without MAD and 97% ± 1% for those with MAD; P = 0.40), even adjusted comprehensively for MVP characteristics (P = 0.80) and accounting for time-dependent mitral surgery (P = 0.60). During follow-up, 170 patients had clinical arrhythmic events (ventricular tachycardia, n = 159; arrhythmia ablation, n = 14; cardioverter-defibrillator implantation, n = 14; sudden cardiac death, n = 3). MAD was independently associated with higher risk for arrhythmic events (adjusted HR: 2.60; 95% CI: 1.87-3.62; P Conclusions This large cohort with MVP comprehensively characterized shows that MAD is frequent at MVP diagnosis and is strongly linked to advanced myxomatous degeneration. The presence of MAD was independently associated with long-term excess incidence of clinical arrhythmic events. However, within the first 10 years post-diagnosis, MAD was not linked to excess mortality, and although reassurance should be provided from the survival point of view, careful monitoring for arrhythmias is in order for MAD.

Objectives The phenomenon of systolic anterior motion (SAM) of the mitral valve (MV) was discovered 50 years ago, but to date only a few studies have identified risk factors for SAM following mitral repair. There are limited data on the necessity of surgical reintervention on the MV once SAM is discovered by intraoperative transesophageal echocardiography. We sought to identify predictors of SAM in a large cohort of consecutive patients, assess the rate of early reintervention on the MV to address SAM, and follow the progression of SAM postdischarge. Methods Analysis of electronically stored echocardiographic exams of adults who underwent MV repair in a recent decade. Results Following MV repair, the incidence of SAM immediately after cardiopulmonary bypass was 13% (98 of 761 patients). Multivariable analysis revealed several preoperative risk factors of SAM development and progression, including a lower ratio of anterior to posterior leaflets heights, younger age, lower end-systolic left ventricular volume, presence of bileaflet prolapse, and male sex. SAM was managed conservatively in 91 patients (93%) and surgically in 7 patients (7%). In a majority of patients (70 of 98 patients [71%]) SAM resolved before hospital discharge. Conclusions Transesophageal echocardiography findings associated with SAM were excessive height of posterior to anterior mitral leaflet, smaller left ventricular end-systolic volume, and bileaflet prolapse. Conservative management of SAM was usually successful, and persistent hemodynamically significant SAM was uncommon. Prophylactic modification of the surgical technique to avoid SAM seems unnecessary for all but those at highest risk for developing SAM.

Objective To examine contemporary clinical differences between men and women with hemodynamically significant chronic aortic regurgitation (AR). Patients and Methods We retrospectively identified 1072 consecutive patients with moderate to severe or severe AR diagnosed between February 21, 2004, and April 29, 2019. Echocardiographic data, aortic valve surgical intervention (AVS), and all-cause death were analyzed. Results At baseline, the 189 women in the study group were older than the 883 men (mean ± SD age, 64±18 years vs 58±17 years), had more advanced symptoms, and had larger left ventricular end-systolic dimension index (LVESDi) (all P Conclusion In contemporary practice, women with AR continue to exhibit an overall survival penalty not related to female sex but to late referral markers, including more advanced symptoms, larger LVESDi, and less AVS. Nonetheless, women in our study exhibited outstanding post-AVS left ventricular remodeling and had good post-AVS survival, a step forward toward closing the sex-related mortality gap. The high percentage of LVESDi of 20 mm/m2 or greater in asymptomatic women represents a window of opportunity for advanced-symptom prevention and timely AR surgical correction that may close the mortality gap.

Objectives This study sought to compare aortic stenosis (AS) progression rates, AS-related cardiac damage (AS-CD) indicator incidence and determinants, and survival between patients with tricuspid aortic valve (TAV)-AS and those with bicuspid aortic valve (BAV)-AS. Background Differences in AS progression and AS-CD between patients with BAV and patients with TAV are unknown. Methods We retrospectively studied consecutive patients with baseline peak aortic valve velocity (peakV) ≥2.5 m/s and left ventricular ejection fraction ≥50%. Follow-up echocardiograms (n = 4,818) provided multiparametric AS progression rates and AS-CD. Results The study included 330 BAV (age 54 ± 14 years) and 581 patients with TAV (age 72 ± 11 years). At last echocardiogram (median: 5.9 years; interquartile range: 3.9 to 8.5 years), BAV-AS exhibited similar peakV and mean pressure gradient (MPG) as TAV-AS, but larger calculated aortic valve area due to larger aortic annulus (p Conclusions In this cohort, TAV-AS and BAV-AS progression rates were similar. Rapid progression did not affect survival and was determined by cardiac risk factors for BAV-AS (particularly in patients with BAV

Functional mitral regurgitation (MR) describes valve leakage in the absence of disease or damage to the mitral leaflets or subvalvular apparatus. Significant, new functional MR after cardiopulmonary bypass (CPB) may result from a number of intraoperative processes, including left ventricular (LV) ischemia and enlargement, left atrial enlargement secondary to increased filling pressure, and systolic anterior motion of the mitral valve after mitral repair. Assessment of new MR after CPB is important because it may direct hemodynamic maneuvers or prompt reinitiation of CPB if surgical intervention is deemed necessary. Described extensively in the electrophysiology literature but underreported as a cause of MR after CPB, LV dyssynchrony represents another possible mechanism of functional MR, in which resynchronization of conduction via pacing maneuvers may prove beneficial. Herein, a series of 4 patients in whom new MR was found after non-mitral valve cardiac surgery in the setting of normal LV systolic function is presented, and LV dyssynchrony is proposed as a major contributing factor. The findings suggested that the concomitant observation of new or worsened functional MR, together with normal global and regional LV systolic function, should lead the clinician to consider ventricular dyssynchrony as a possible cause. Attempts to improve or alter ventricular conduction should be considered before contemplating a return to CPB for mitral valve intervention.

Aortic valve stenosis (AS) results in significant cardiovascular morbidity and mortality. To guide patient stratification and treatment in AS, echocardiographically defined cardiac damage classes h...

Objective To describe the clinical history of patients with a wide age range diagnosed with bicuspid aortic valve (BAV) and no surgical indication and to evaluate the long-term outcome of patients with BAV referred for elective surgery. Patients and Methods Between 2005 and 2017, 350 consecutive patients with no surgical indication (surveillance group, mean age 53±16, 71% men) and 191 with a surgical indication (surgical group, mean age 59±13, 71% men) were prospectively included. Median follow-up was 80 (32 to 115) months. Results In the surveillance group, the 5-year and 10-year survival rates were 93±1% and 89±2%, respectively, with a relative survival of patients with BAV compared with an age- and sex-matched control population of 98.7%. During follow-up, the cumulative 10-year incidence of aortic valve and aorta surgery was high; of 35±4%, the incidence of native valve infective endocarditis (IE) of 0.2% per patient-year, and no cases of aortic dissection were observed. In the surgical group, the 5-year and 10-year survival rates were 97±1% and 89±3%, respectively, with a relative survival of 99.4% compared with the general population. The incidence of IE was 0.4% per patient-year, and no cases of aortic dissection were observed. Conclusion This regional cohort shows that the 10-year survival rates of patients with BAV and a wide age range, but mostly middle-aged adults, were similar to those of the general population with a very low rate of complications. Adherence to prophylactic surgical indications and younger age might have contributed to this lack of difference.

This study aims to investigate the association of wall shear stress (WSS) and aortic strain with circulating biomarkers including matrix metalloproteinases (MMP), tissue inhibitors of metalloproteinase (TIMP), and exosomal level of microRNA (miRNA) in ascending aortic aneurysms of patients with bicuspid or tricuspid aortic valve.A total of 76 variables from 125 patients with ascending aortic aneurysms were collected from (1) blood plasma to measure plasma levels of miRNAs and protein activity; (2) computational flow analysis to estimate peak systolic WSS and time-average WSS (TAWSS); and (3) imaging analysis of computed tomography angiography to determine aortic wall strain. Principal component analysis followed by logistic regression allowed the development of a predictive model of aortic surgery by combining biomechanical descriptors and biomarkers.The protein activity of MMP-1, TIMP-1, and MMP-2 was positively correlated to the systolic WSS and TAWSS observed in the proximal ascending aorta (eg, R = 0.52, P.001, for MMP-1 with TAWSS) where local maxima of WSS were found. For bicuspid patients, aortic wall strain was associated with miR-26a (R = 0.55, P = .041) and miR-320a (R = 0.69, P.001), which shows a significant difference between bicuspid and tricuspid patients. Receiver-operating characteristics curves revealed that the combination of WSS, MMP-1, TIMP-1, and MMP-12 is predictive of aortic surgery (area under the curve 0.898).Increased flow-based and structural descriptors of ascending aortic aneurysms are associated with high levels of circulating biomarkers, implicating adverse vascular remodeling in the dilated aorta by mechanotransduction. A combination of shear stress and circulating biomarkers has the potential to improve the decision-making process for ascending aortic aneurysms to a highly individualized level.

ObjectiveTo comprehensively explore contemporary differences between bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) patients with chronic haemodynamically significant aortic regurgitation (AR).MethodsConsecutive patients with chronic ≥moderate-severe AR from a tertiary referral centre (2006–2017) were included. All-cause mortality, surgical indications and aortic valve surgery (AVS) were analysed.ResultsOf 798 patients (296 BAV-AR, age 46±14 years; 502 TAV-AR, age 67±14 years, p20 mm/m2; similar thresholds were observed for BAV-AR patients.ConclusionBAV-AR patients were two decades younger than TAV-AR and underwent AVS more frequently, resulting in a considerable real-life survival advantage for BAV-AR that was determined primarily by age and not valve anatomy. Pragmatically, regardless of valve anatomy, patients with haemodynamically significant AR and age >50–55 years require a low-threshold for surgical referral to prevent symptom development where LVEF 20 mm/m2 seem appropriate referral thresholds.

Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. BAV is a heterogeneous disease with a wide array of presentations at various ages, depending on the degree of aortic valve dysfunction, aortic dilation and presence of associated lesions. Aortic valve stenosis and/or regurgitation are the primary indications for intervention in children and adolescents with BAV. Although a majority of young patients with BAV also have some aortic dilation, interventions on the aorta are very rare during this time frame. Children and adolescents with BAV benefit from comprehensive assessment of their risk profile to determine follow-up surveillance intervals, sports recommendations, and timing of surgical intervention. The morphologic phenotype of BAV is important to identify, as it may predict future complications and prognosis.

Etiology, mechanisms, and survival of mitral regurgitation (MR) plus hemodynamically-significant chronic aortic regurgitation (AR) are mostly unknown.The purpose of this study was to investigate the prevalence, mechanisms, etiologies, and survival impact of coexistent ≥ moderate MR in AR patients.Consecutive patients with ≥ moderate-severe AR were retrospectively identified between 2004 and 2019.Of 1,239 eligible patients (61 ± 18 years, 80% men), 1,072 (86%) had pure AR, and 167 (14%) had AR + MR (9% functional mitral regurgitation [FMR] [84% nonischemic] and 5% organic mitral regurgitation [OMR] [62% degenerative]). At baseline transthoracic echocardiogram, pure AR versus AR + OMR versus AR + FMR exhibited differences in age (59 ± 18, 62 ± 16, and 73 ± 14 years, respectively), female sex (18%, 27%, and 39%, respectively), symptoms (36%, 41%, and 64%, respectively), atrial fibrillation (5%, 17%, and 36%, respectively), left ventricular (LV) ejection fraction (59%, 58%, and 46%, respectively), LV end-systolic dimension and volume index, ≥ moderate tricuspid regurgitation (TR) (7%, 35%, and 53%, respectively), and right ventricular systolic pressure (32 ± 11, 45 ± 15, and 50 ± 14 mm Hg, respectively), all p 0.0001. After a median follow-up of 5.2 years (interquartile range: 2.2 to 10.0 years) and adjusting for demographics, New York Heart Association functional class, aortic valve surgery, LV ejection fraction, LV end-systolic dimension and volume index, presence of FMR was independently associated with all-cause mortality (p ≤ 0.004). Compared with pure AR, AR + MR + TR exhibited the highest adjusted risk of death (2.4-fold; p 0.0001). When compared with expected population survival, excess mortality risks of pure AR, AR + OMR, and AR + FMR were 1.25-fold, 1.76-fold, and 2.34-fold, respectively (all p ≤ 0.02).In hemodynamically significant AR, coexistent MR is not uncommon (approximately 14%) and mostly comprises FMR and less commonly OMR. As compared with pure AR, AR + MR + TR exhibit the largest mortality risk. Both AR + OMR and AR + FMR carry a survival penalty compared with the general population, but AR + FMR is associated with the largest excess mortality and represents an advanced stage within the AR clinical spectrum.

The most common complication of patients with bicuspid aortic valve (BAV) is aortic valve surgery ([1][1]). Hemodynamically significant (moderate-severe to severe) aortic regurgitation (AR) is more common in younger men with BAV ([1][1]) and is classified as Stage C (asymptomatic) or D (symptomatic